Unfortunately for the first several years the only way to get a good eye exam is under sedation. The exam itself takes minutes yet the whole process is hours.
the day of an EUA (Exam Under Anesthesia)
Because Cleveland is a 2 hour drive for us we always make sure we leave the house with at least a half hour to spare. Will has to stop solid foods 8 hours prior to surgery, formula 6 hours, breastmilk 4, and Pedialyte 2 hours before. A 2 hour drive with a hungry baby is never fun. I usually have to pump at least once in the car. Once we get to the surgery center we check in and wait for them to call us back. The wait can be anywhere between 10 minutes to 2 hours. Jon and I take turns soothing a hangry Will as much as possible.
Once we are called back they get started right away with eye drops. One of us holds him down while the nurse administers the drops We have to strip him and put him in a hospital gown which is when the gravity of the day really sinks in. As hard as it is holding a crying baby, handing him over and watching him walk away in someone else's arms to the operating room is by far the worst feeling ever.
Just before they take Will back the whole operating team meets with us to go over the plan for the day and answer any last questions. However we rarely know the extent of the plan until Will is under and they get a closer look. Those last moments with him are priceless I always fear the worst. Walking out of the room without my baby to wait for any news is like having your stomach up in your throat, and your head in a blender. You feel completely helpless and scared as your purpose in life is out of your hands.
EUA #1
The main goal of this first exam was to get and angiogram image of both eyes and treat accordingly. Based on what little they had seen in clinic they assumed they would likely be doing laser therapy, scleral buckle, and a vitrectomy.
The basis of Will's condition is a genetic mutation causing the blood vessels that supply the retina to not form properly. Once they saw his eye's closer it was unlike anything they had ever seen before. Will only had a small fraction of his retina in tact. He had huge hemorrhages in both eyes. So many blood vessels didn't form right they were leaking large amounts of blood in the retina causing pools of blood.
Laser Therapy - The purpose of this treatment is to stop the bleeding. It essentially kills off the deformed blood vessels to stop the hemorrhaging.
Scleral Buckle - with the lack of blood supply the retina pulls away from where it should develop and gets twisted up. Without surgical intervention the retina will continue to be pulled further and the tension to increase. The idea behind the scleral buckle is to take the pressure off of the retina so it's no longer being pulled and has a chance to relax in place. Will's retina was being pulled nasally in both eyes.
Vitrectomy - This was presented to us as a worst case scenario. They remove the vitreous from the eye. The way I understand this is that the vitreous plays a key role in the overall problem of the tension in the eye. Removing it helps relieve the tension and gives them the room to make more drastic repairs to the retina such as reattachment, scar tissue removal, and macular repair.
The hope was that all Will's eyes needed was lasering and things would stabilize with just that. Based on what little we knew however we were expecting they would do all 3 procedure as we knew his eyes were in bad shape
What they saw changed everything!
If proportionally your retina is supposed to be the size of North America Will's retina is the size of Dayton Ohio or smaller. Our surgeon refers to it as his little island of a retina. Dr. Sears (retinal surgeon) Explained to us that things were not only being pulled nasally but had twisted to a point surgery he felt would do more harm than good. He explained that it would be impossible to perform any surgery without severing the small bit of retina that was still attached. The best option we had was to just do lasering of the blood vessels and hope that the small little island of retina would hold!
If Will was going to have any hope of vision it would be peripheral. Our hopes of 20/200 vision were gone. Our hopes now were just in any type of peripheral vision. If he could see peripherally it would likely be very cloudy and abstract. Our hope was that he would be able to walk and navigate without a cane. At this pointe we knew he could still see some as he would react to the light being turned on in his room and walking in and out of shade on a sunny day. We were hoping he would keep whatever he had! With this disease there is no undoing the damage that had already occurred but maybe we could preserve something...
